Progressive cognitive dysfunction in spinocerebellar ataxia type 3

摘要: Background Although it is well established that there cognitive dysfunction in spinocerebellar ataxia type 3 (SCA3), unknown whether cognition deteriorates with disease progression. We therefore prospectively studied function patients SCA3. Methods Eleven SCA3 were assessed using an extensive neuropsychological test battery and retested after 3.5 ± 0.4 years. Results In addition to motor control, verbal learning figural memory deteriorated significantly during the follow-up period. An increase depressive symptoms was not observed. Conclusions The observation abilities progression suggests integral part of SCA3. Because applied tests for did require responses, decline cannot be attributed progressive cerebellar ataxia. The deterioration can explained either by extracerebellar pathology or disruption cerebellar-cerebral circuitries. © 2013 International Parkinson Movement Disorder Society